{"entity":{"id":"sertoli-cell-tumour","kind":"cancer","name":"Sertoli cell tumour of the testis","aka":["Sertoli cell tumour","Sertoli cell tumor","Sertoli cell tumour, not otherwise specified","Large cell calcifying Sertoli cell tumour","Androblastoma of the testis"],"tldr":"Sertoli cell tumour is a rare testicular tumour arising from the cells that support sperm production. Most are benign and cured by removing the testis; about one in ten spread, and there is no good treatment for those. A calcifying form occurs in boys with Peutz-Jeghers or Carney syndromes, usually with breast enlargement.","summary":"Sertoli cell tumours are sex cord-stromal tumours of the testis; the WHO classification separates the not otherwise specified type from the large cell calcifying and the intratubular large cell hyalinising types (Moch 2016). In the series of 60 tumours not otherwise specified, presentation was usually a slowly enlarging painless mass, all were unilateral, size ranged from 0.3 to 15 cm (mean 3.6), nuclear atypia was absent or mild in 54, and four patients had metastases at presentation (Young 1998). Exon 3 mutations of CTNNB1 with nuclear beta-catenin were found in 10 of 14 tumours, linking the tumour to WNT pathway activation (Am J Surg Pathol 2014). In boys, intratubular Sertoli cell proliferations and large cell calcifying tumours occur with Peutz-Jeghers syndrome and gynaecomastia (Am J Surg Pathol 2001).\n\nHow it differs from its parent: like Leydig cell tumours it is not a germ cell tumour, does not respond to cisplatin chemotherapy, does not raise the germ cell markers and is usually benign; malignancy is judged from size over 5 cm, necrosis, vascular invasion, atypia and mitoses.\n\nHow common: no incidence figure in the sources read.\n\nTreatment: orchidectomy (testis-sparing in syndromic boys with bilateral tumours); retroperitoneal lymph node dissection for malignant features; no effective systemic therapy for metastatic disease.","asOf":"2026-09-24","wikipedia":"https://en.wikipedia.org/wiki/Sertoli_cell_tumour","links":[{"label":"NCI PDQ: testicular cancer treatment","url":"https://www.cancer.gov/types/testicular/treatment"},{"label":"Moch 2016, European Urology: the 2016 WHO classification of urinary and male genital tumours, part A","url":"https://doi.org/10.1016/j.eururo.2016.02.029"},{"label":"Young 1998, Am J Surg Pathol: Sertoli cell tumours of the testis, not otherwise specified, 60 cases","url":"https://doi.org/10.1097/00000478-199806000-00008"},{"label":"Am J Surg Pathol 2014: frequent CTNNB1 mutation and nuclear beta-catenin in Sertoli cell tumours of the testis","url":"https://doi.org/10.1097/pas.0b013e31829cdbc6"},{"label":"Am J Surg Pathol 2001: intratubular Sertoli cell proliferations of the infantile testis in Peutz-Jeghers syndrome","url":"https://doi.org/10.1097/00000478-200110000-00003"}],"tags":["subtype-page","wave4","testicular","rare"],"related":["testicular","leydig-cell-tumour","granulosa-cell-tumour","childhood-cancers"],"cancers":[],"sections":[],"technologies":[],"targets":[],"drugs":[],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"genitourinary","burden":"Rare: the defining series holds 60 tumours not otherwise specified, patient age 15 to 80 (mean 45), four with metastatic disease at presentation (Young 1998). No registry figure was found in the sources read.","subtypes":[],"biomarkers":["Inhibin, calretinin and nuclear beta-catenin; germ cell markers negative","CTNNB1 exon 3 mutation","Size, necrosis, vascular invasion, atypia and mitotic count (malignancy risk)","Peutz-Jeghers or Carney syndrome features in boys"],"standardOfCare":[{"setting":"All cases","approach":"Orchidectomy, testis-sparing in syndromic boys; node dissection for malignant features; no effective systemic therapy for metastatic disease.","refs":["testicular"]}],"stateOfArt":[],"history":[],"pipeline":[],"openProblems":[],"parent":"testicular"},"route":"/cancers/sertoli-cell-tumour/","neighbours":{"cancer":[{"id":"granulosa-cell-tumour","kind":"cancer","name":"Adult granulosa cell tumour of the ovary","route":"/cancers/granulosa-cell-tumour/"},{"id":"childhood-cancers","kind":"cancer","name":"Childhood cancers (all types)","route":"/cancers/childhood-cancers/"},{"id":"leydig-cell-tumour","kind":"cancer","name":"Leydig cell tumour of the testis","route":"/cancers/leydig-cell-tumour/"},{"id":"testicular","kind":"cancer","name":"Testicular germ cell tumours","route":"/cancers/testicular/"}]}}